CASE

42 year old right handed Hispanic female with a known PMH of DM, HTN,
bilateral blindness and right facial droop at baseline. She has had prior right
common carotid angioplasty and stenting at outside hospital. H/o prior
angioplasties of her Left CCA and ICA at this hospital. Presents to clinic today
for follow-up of repeat MRA imaging of her head and neck performed on
9/15/15. The patient reports feeling "great" since last angioplasty of her left
common carotid and left internal carotid arteries on 9/25/13.
She reports no new symptoms since last angioplasty. She does state that
she has occasional episodes of dizziness. These episodes occur
approximately once a month last up to 5 minutes but resolve spontaneously
with rest. She also reports generalized fatigue and she easily tires that this
has been ongoing for several years. Her only other complaint is occasional
insomnia for which she is requesting if she can have some medications to
help her sleep. She does not complain of any new onset of any focal
neurological deficits but she does have occasional numbness in her
fingertips bilaterally that also resolve spontaneously.

CASE
Allergies: NKDA
PMH: Hypertension, DM, Bilateral blindness
PSH: angioplasty and stenting of the right internal carotid
artery at outside hospital. Angioplasty of the Left Common
Carotid on 9/25/13. h/o cholecystectomy and
appendectomy
Medications: amlodipine 5 mg tablet, Aspirin-81 mg
tablet,delayed release, azathioprine 50 mg tablet,
benazepril 20 mg tablet, clopidogrel 75 mg tablet,
pravastatin 40 mg tablet, prednisone 5 mg tablet,
VITAMIN B COMPLEX & VIT C NO.3
Family History: + Diabetes
Social History: Denies tobacco, alcohol, or drug use

CASE
Physical Exam:
VITALS: 172/108 (right calf), HR 94, Temp 97.0
Gen: NAD. + Central obesity
Neck: + Left carotid bruit
CV: RRR, no murmurs
Resp: CTA bl, no wheezes
Neuro Exam:
Mental status: Alert and oriented 3. Speech is fluent without any dysarthria.
Cranial nerves: Total bilateral blindness. Left pupil 8mm and unreactive to light.
Opacity of the right eye. EOMI without nystagmus. Sensation to light touch is
intact bilaterally. Right sided droop and flattening of the nasolabial fold.
Motor: Strength is 5/5 in all extremities. Pronator drift is negative
Sensory: Sensation light touch is intact in all extremities. Negative rhomberg.
Reflexes: 2/4 throughout
Coordination: Finger to nose test is smooth and coordinated
Gait: No ataxia noted

CASE
Radiology:
MRA of the head on 9/15/15 show occlusion to near
complete occlusion of the previously stented left
common carotid artery with reconstitution distally and
patency throughout the left internal carotid artery.
The proximal 4 cm right common carotid artery
demonstrates possible paucity of flow and likely
occluded with reconstitution of flow distally within the
right common carotid artery and distal internal carotid
artery.
Chronically occluded L vertebral artery
No stenosis of intracranial vessels

 Differential Diagnosis?

VASCULITIS
Large Vessel
Vasculitis
Takayasu Arteritis
Giant cell arteritis

Medium Vessel
Vasculitis
Polyarteritis Nodosa
Kawasaki Disease

Small Vessel Vasculitis

ANCA associated
Microscopic polyangitis
Wegners Granulomatosis
Eosinophilic Granulomatosis
(Churg Straus)

Immune Complex
Anti-glomerular Basement
membrane disease
(Goodpastures)
Cryoglobulineimic vasculitis
IgA Vasculitis (HenochSchonlein purpura)

Bechets Disase

VASCULITIS
D AV I D N A C I O N A L E S , P G Y 1

OBJECTIVES
1. Case presentation
2. Takayasu Arteritis
Etiology
Definition
Differential Diagnosis
Work-up
Treatment
3. Other Vasculitic conditions

TAKAYASU ARTERITIS

DEFINITION
TAKAYASUS ARTERITIS
An inflammatory and stenotic disease of medium and
large sized arteries characterised by a strong prediliction
for the aorta and its branches .

AORTIC ARCH SYNDROME

PULSELESS DISEASE.

SUBTYPES
Primarily affects the aorta and its primary
branches

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INCIDENCE & PREVALENCE

Most commonly found in Asia?
~150 new cases in Japan each year

Annual incidence rate 1-3

cases/million/year in the US and Europe
combined
Prevalent in adolescent and young women
Typical age of onset 10-40 years
80-90% of cases in females

PATHOGENESIS
Poorly understood
Likely cell mediated
Affects all 3 layers
Giant cells and granulomatous inflammation in the media
Destruction of the elastic lamina and muscular media

Scarring of the adventitia

Proliferation of the intima

CLINICAL FEATURES
Early signs
Unspecific (due to
inflammation)
Fatigue
Weight loss
Chronic low grade fevers

Vascular symptoms
Claudication
Subclavian steal
Myalgias/Arthralgias
Skin manifestations (erythema
nodosum)

Cerebral (Carotids or Vertebral)

Stroke
Blindness is a late sign

Pulmonary

Chest pain
Dyspnea
Hempotysis
PH

Renal/Mesenteric
Aortic Regurgitation

PHYSICAL EXAM
Particular attention to peripheral pulses, blood
pressure in all 4 extremities, and an
ophthalmologic examination.
The most discriminatory finding is a systolic blood
pressure difference (>10 mm Hg) between arms.
Hypertension due to renal artery involvement is
found in approximately 50% of patients.
Absent or diminished pulses are the clinical
hallmark of Takayasu arteritis, but pulses are
normal in many patients and upper limbs are
affected more often Than lower limbs.

PHYSICAL EXAM
Carotidynia (Unilateral neck pain) may be present.
Bruits are often noted.
Aortic regurgitation is a common finding.
Ophthalmologic examination may show retinal
hemorrhages, cotton-wool exudates, venous
dilatation and beading, microaneurysms of
peripheral retina, optic atrophy, vitreous
hemorrhage, and classic wreathlike peripapillary
arteriovenous anastomoses (extremely rare).
Skin changes resembling erythema nodosum or
ulcerating nodular lesions may be seen.

DIAGNOSIS
Labs
Acute phase reactants

ESR
CRP
Alpha-2-globulin
Hypoalbuminemia

No specific antibody marker is indicative of Takayasu

Negative
Negative
Negative
Negative

ANA
ANCA
anti-DNA
anti-phospholipid

DIAGNOSTIC IMAGING
X-ray
CT/MRI
Diagnostic Angiography (gold-standard)
Doppler US

DIFFERENTIAL
Giant Cell Arteritis
Aortic Coarctation
Atherosclerosis
Bechets Disease
Buergers Disease (Thromboangitis
obliterans)
Kawasaki Disease
Rheumatoid Arthritis
Sarcoidosis

CLINICAL CRITERIA

TREATMENT
Medical Management
Glucocortocoids are first line but only about 50% are
responsive
Immunosuppressants
Methotrexate
Azathioprene

Surgical
Angioplasty
Bypass graft

GIANT CELL ARTERITIS

VASCULITIS
Large Vessel
Vasculitis
Takayasu Arteritis
Giant cell arteritis

Medium Vessel
Vasculitis
Polyarteritis Nodosa
Kawasaki Disease

Small Vessel Vasculitis

ANCA associated
Microscopic polyangitis
Wegners Granulomatosis
Eosinophilic Granulomatosis
(Churg Straus)

Immune Complex
Anti-glomerular Basement
membrane disease
(Goodpastures)
Cryoglobulineimic vasculitis
IgA Vasculitis (HenochSchonlein purpura)

Bechets Disase

DEFINITION
Giant Cell Arteritis
An inflammatory disease of medium and large sized
arteries.
Can affect the aorta and other great vessels
Most commonly targets the cranial branches of the aortic
arch, especially the superficial temporal artery.

Temporal Arteritis

EPIDEMIOLOGY
Most common systemic vasculitis
Affects individuals age >50 with steep
increase in prevalence >70 years
Females > Males

CLINICAL FEATURES
Constitutional symptoms
+Fever, fatigue, weight loss

Headache
Jaw claudication
Amaurosis Fugax
Vision loss
Complication of untreated/late treatment
Up to 20% of cases

TREATMENT
Glucocorticoids is the mainstay of
treatment
Early initiation is key in suspected cases
Confirmation with temporal artery biopsy
Followed by close monitoring for further
complications

BECHETS DISEASE

BECHETS
Vasculitis that can affect any sized artery
Prevalence: M>F
Most common in Middle eastern and Asian
populations
One of the leading causes of blindness in
Japan

DIAGNOSTIC CRITERIA
Pulmonary vessels most commonly affected
No specific diagnostic labs
3 points or greater (86-97% sensitivity) :
Genital aphthosis Two points
Ocular lesions (anterior uveitis, posterior uveitis, or retinal
vasculitis) Two points
Oral aphthosis One point
Skin lesions (pseudofolliculitis or erythema nodosum) One point
Vascular lesions (superficial phlebitis, deep vein thrombosis,
large vein thrombosis, arterial thrombosis, or aneurysm) One
point
Pathergy One point

PATHERGY TEST

CLINICAL PEARLS
Clinical diagnosis
Eye involvement (conjunctiva) but does
not cause blindness
Can lead to aortic aneurysm if left
untreated (5x risk)
Treat with IVIG, not steroids

KAWASAKI SYNDROME

KAWASAKI
Mucocutaneous lymph node syndrome
Most common vasculitis syndrome in
children
Can rarely occur in adults

DIAGNOSTIC CRITERIA
Unexplained fever > 5 days
Any 4 out of 5 symptoms:
Bilateral bulbar conjunctival injection
Oral mucous membrane changes, including injected or
fissured lips
Peripheral extremity changes, including erythema of
palms or soles, edema of hands or feet (acute phase)
Polymorphous rash
Cervical lymphadenopathy (at least one lymph node >1.5
cm in diameter

CLINICAL PEARLS
Clinical diagnosis
Eye involvement (conjunctiva) but does
not cause blindness
Can lead to aortic aneurysm if left
untreated (5x risk)
Treat with IVIG, not steroids

SUMMARY
In suspected Takayasu arteritis or any other
vasculitic conditions:
Early treatment with corticosteroids or other immune
modulating drugs is key
Inflammatory work up
Clinical diagnosis, good history and physical exam
Systemic disease, many co-morbidities
Close follow up

CASE 1
A 19 year old woman from Thailand had reported to family members
felling unwell for weeks with fevers and malaise. She had a history of
erythema nodosum and mild anemia that was under investigation.
Other symptoms she had previously reported included pain in the
calves whenever she walked more than approximately 50 meters
without rest. One morning, she is found collapsed on the floor of her
apartment, unresponsive. She is taken to an emergency department
where a CT scan of the brain showed an acute infarct involving the
entire territory of the left MCA. MRI of the brain also shows an
extensive right lateral medullary infarct. Angiogram shows significant
disease of the aortic arch, bilateral severe narrowing of the sublclavian
arteries and left carotid and right vertebral occlusion. What is the
most likely diagnosis in this patient?

A. Primary angitis of the CNS

B. Polyarterisis nodosa
C. Takayasu arteritis
D. Temporal arteritis
E. Microscopic polyangitis

CASE 1
A 19 year old woman from Thailand had reported to family members
felling unwell for weeks with fevers and malaise. She had a history of
erythema nodosum and mild anemia that was under investigation.
Other symptoms she had previously reported included pain in the
calves whenever she walked more than approximately 50 meters
without rest. One morning, she is found collapsed on the floor of her
apartment, unresponsive. She is taken to an emergency department
where a CT scan of the brain showed an acute infarct involving the
entire territory of the left MCA. MRI of the brain also shows an
extensive right lateral medullary infarct. Angiogram shows significant
disease of the aortic arch, bilateral severe narrowing of the sublclavian
arteries and left carotid and right vertebral occlusion. What is the
most likely diagnosis in this patient?

A. Primary angitis of the CNS

B. Polyarterisis nodosa
C. Takayasu arteritis
D. Temporal arteritis
E. Microscopic polyangitis
Comprehensive Review in Clinical Neurology, Cheng-Ching, page 683

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November 30, 2015.)
Hunder, Gene. Clinical features and diagnosis of Takayasu arteritis. In: UpToDate,
Post TW (Ed), UpToDate,Waltham, MA. (Accessed on November 30, 2015.)
Hunder, Gene. Treatment of Takayasu arteritis. In: UpToDate, Post TW (Ed),
UpToDate,Waltham, MA. (Accessed on November 30, 2015.)
Merkel, Peter. Overview of and approach to the vasculitides in adults. In: UpToDate,
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Smith, Ellison, Yazici, Yousuf. Clinical manifestations and diagnosis of Behets
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http://www.hopkinsvasculitis.org/types-vasculitis/behcets-disease/
http://www.the-rheumatologist.org